Pulmonology Flashcards
Primary ciliary dyskinesia genetics
Homozygous mutation in DNAH11
Primary ciliary dyskinesia symptoms
- History of neonatal respiratory issues
- Chronic daily nasal drainage
- Chronic wet cough that is worse in the morning
- Chronic otitis media
- Recurrent bronchitis/pneumonia
- Bronchiectasis
- Male infertility
Situs inversis totalis, chronic sinusitis, bronchiectasis
Kartagener syndrome
Infant or toddler with nonproductive cough, expiratory wheeze, and decreased breath sounds
Foreign body aspiration
- Toddlers aspirate food (hot dogs, popcorn)
- Older kids aspirate objects
- Cough of SUDDEN ONSET
- Often in the right mainstem bronchus
- Diagnose: inspiratory/expiratory films, airway fluoroscopy, bronch to retrieve the object
Symptoms/diagnosis of swallowing dysfunction
- Cough/wheezing after feeds
- Confirm with barium swallow study with video fluorscopy
- Tx: thickened feeds, feeding therapy, maybe NG
Recurrent wheezing in an infant that increases with feeding and neck flexion
Vascular ring or other things compressing the trachea
- Can also have stridor and/or dyspnea during feeding
Hemoptysis, iron deficiency anemia, and diffuse pulmonary infiltrates
Hemosiderosis
Treatment for congenital malformations of the lung
Surgical removal
- Present with recurrent respiratory symptoms or can be incidental finding on CXR
Causes of clubbing
- Cyanotic heart disease
- Chronic lung disease
- Cirrhosis of the liver
Technical term: hypertrophic pulmonary osteoarthropathy
Lab definitions of respiratory failure
- PaCO2 > 50
- PaO2 < 50
CF sweat test diagnostic values
< 30 is negative
30-60 is intermediate
> 60 is positive
Nasal polyps, rectal prolapse, meconium ileus –> what test do these people need?
Sweat test to rule out CF
Pleural fluid characteristics associated with empyema
- Purulent fluid
- Cell count: > 10,000 WBC
- pH < 7.1
- LDH > 1000
- Glucose < 50% serum glucose
Definition of pulsus paradoxus
Difference in BP of > 20 mmHg between inspiration and expiration –> this suggests pulmonary or cardiac issues
Signs of chronic hypoxemia
- Increased hematocrit: headaches, joint pain, clots (PEs), hemoptysis
- Increased risk of bleeding due to shortened platelet life
Symptoms of hypercarbia vs hypoxia
- Hypoxia: cyanosis, depressed sensorium
- Hypercarbia: flushing, agitation, headaches –> cerebral vasodilation
Indications for home apnea monitor
- Severe BRUE (requiring CPR)
- Symptomatic apnea of prematurity
- Central hypoventilation syndrome
Symptoms of hypoxia
Cyanosis, depressed sensorium
Symptoms of hypercarbia
Flushing, agitation, headaches - due to elevated CO2 leading to cerebral vasodilation
Symptoms of chronic hypoxemia
- Elevated hematocrit –> headaches, joint pain, clots, hemoptysis
- Respiratory drive is driven by hypoxemia (rather than hypercapnia) so give patients the lowest amount of oxygen they need otherwise could lead to respiratory arrest
What does pulse ox measure
- Differential light abosrption of oxyhemoglobin and deoxyhemoglobin so estimates the saturation of hemoglobin with oxygen
Carbon monoxide poisoning and pulse ox
- Falsely elevated pulse ox reading due to carboxyhemogobin absorbing light in the same wavelength as oxyhemoglobin
Definition of cyanosis
- Central blueish discoloration of the skin due to poorly oxygenated blood
- Can be seen at 5 g/dL of desaturated hemoglobin so more readily seen at higher hemoglobin leels
Cause and symptoms of methemoglobinemia
- Due to iron in the hemoglobin molecule being oxidized ferric state so can’t unload oxygen –> cyanosis
- Fatigue, dizziness, nausea, arrhythmias, seizures, altered mental status
- Pulse ox will be around 85% but this is not a reliable/true reading
- Treat with methylene blue
Loud barking cough that can be produced on command and disappears with sleep
Psychogenic cough
Initial workup for chronic cough
Sweat chloride, TB skin test, CXR, spirometry if > 6 years old
Diagnostic cutoff for sweat chloride test
> 60 mEq — this is the gold standard for diagnosis of CF
Genes and inheritence of CF
- Autosomal recessive
- CFTR gene
- Odds of a healthy sibling of someone with CF being a carrier is 2/3
- General caucasian carrier rate is 1/25
Newborn screening limitations/follow up for CF
- Newborn screen is only 95% sensitive
- Only 1/20 infants with an abnormal NBS for CF have CF
- Need a sweat test
Main GI symptoms of CF
- Newborn: meconium ileus (polyhydramnios can be presenting sign), meconium peritonitis (pseudocyst on xray), unconjugated hyperbilirubinemia
- Steatorrhea, fat soluble vitamin deficiency (especially vitamin E)
Bacteria and treatment involved in CF exacerbations
- Staph aureus, H. flu, psuedomonas, Burkholderia cepacia (associated with worsening lung function)
- Tx with aminoglycoside and penicillin (piperacillin)
Newborn with respiratory distress after C-section and oxygen sats lower in lower extremities than in upper extremities
PPHN - can also have a precordial lift or RV heave
Signs of cor pulmonale
- Lower body edema, hepatomegaly, gallop, clubbing
- Often caused by pulmonary hypertension and dis due to RV dysfunction
Chronic sinusitis, bronchiectasis, male infertility
Primary ciliary dyskinesia - autosomal recessive
- Only way to diagnose is with a biopsy
- Associated with situs inversus and Kartagener syndrome
Pleural fluid with triglycerides > 110, elevated lymphocyte count, protein greater than 3
Chylothorax
Plueral fluid with LDH at least 2/3 the serum LDH, protein is 3 or greater, pH < 7.3
Exudative - seen with inflammation - pneumonia, cancer, trauma, inflammatory disease
Pleural fluid with LDH < 2/3 serum concentration, protein < 3, pH > 7.45
Transudative - congestive heart failure
Patient diagnosed with pneumonia initially improves and then worsens again
Think empyema – chest tube, drainage, IV antibiotics
Patient with tachypnea, tachycardia, unilateral decreased breath sounds
- Can also be associated with marijuana smoking
Pneumothorax
- Treat with observation and oxygen if small
- If needed can do needle decompression and/or chest tube if tension
Causes of bronchiectasis
- PCD
- CF
- Immunodeficiency
- Lobar pneumonia
- Aspergillosis, measles, pertussis, TB
- Extrinsic compression
Symptoms and diagnosis for bronchiectasis
- Repeated lower respiratory tract infections, coughing that is worse with change in position
- Dx with CT of the chest
Most common causes of hemoptysis in kids
- Infection (pneumonia, TB)
- CF (bronchiectasis)
- Foreign body aspiration
- Hemosiderosis
Workup for hemoptysis
- pH of the emesis (acidic is stomach, alkaline is lungs)
- CBC, coags
- CXR and direct visualization of the airway
MCC pneumonia in infants (3 weeks through 12 months)
- Chlamydia transmitted during delivery (afebrile, interstitial infiltrates on CXR)
- RSV (late fall, wheezing)
- Parainfluenza (fall through spring)
- Pertussis (paroxysmal cough, no fever)
MCC pneumonia in pre school (1-4 years)
- Viral (RSV, parainfluenza, influenza, rhinovirus, HMPV)
- S. pneumonia (productive cough, can have abdominal pain and vomiting as well, fevers)
MCC pneumonia in school ages
- Mycoplasma pneumonia (low grade fever, CXR with diffuse non focal infiltrates)
- Chlamydophilia
- S. pneumonia (can have empyemas in this age)
- Mycobacterium TB (with risk factors)
- S. aureus (following influenza)
3 most common complications of pneumonia
- Effusions/empyema
- Lung abscess
- Necrotizing pneumonia (tx with vanc/clinda)
Side effects of albuterol
Tremors, tachycardia, hypokalemia
Steroids effects in asthma
Interferes with late response and airway wall edema/inflammation but not early response (bronchoconstriction due to IgE mediation needs beta agonsists)
Xray finding for foreign body aspiration
Unilateral hyperlucency on expiration film
Long term complications of foreign body aspiration
Chronic cough, bronchiectasis, lung asbcess, recurrent pneumonia, unnecessary medications
Cause and implications of hydrocarbon pneumonitis
- In household products
- Can lead to respiratory distress and secondary infections
- Long term risk for lung fibrosis
Hemoptysis, iron deficiency anemia, and diffuse pulmonary infiltrates
Hemosiderosis
- Sx can include fatigue, cough, wheezing, dyspnea, hypoxemia and respiratory failure
Differential diagnosis of recurrent pneumonia
- Aspiration - TE fistula, laryngeal cleft
- Foreign body
- Anatomical malfromation (bronchogenic cyst, CCAM)
- CF
- PCD
Manifestations of CF in infancy
Hypoalbuminemia, anemia, steatorrhea, recurrent pulmonary symptoms, hypokalemia alkalosis
